{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:50:19Z","timestamp":1787028619696,"version":"build-2736575974"},"reference-count":21,"publisher":"MDPI AG","issue":"19","license":[{"start":{"date-parts":[[2021,9,28]],"date-time":"2021-09-28T00:00:00Z","timestamp":1632787200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Primary sclerosing cholangitis (PSC) is a cholestatic liver disease with a variable clinical course that can ultimately lead to end-stage liver disease, cholangiocarcinoma, and the need for liver transplantation. Several prognostic models have been developed to predict clinical outcomes and have been compared to the revised Mayo Risk Score (rMRS). Aim: To conduct a systematic review comparing the rMRS to other non-invasive prognostic tests for PSC. Methods: A systematic review of studies from 2000 to 2020 was performed that compared non-invasive biochemical prognostic models to the rMRS in predicting outcomes in patients with PSC. Results: Thirty-seven studies were identified, of which five studies that collectively included 3230 patients were reviewed. Outcomes included transplant-free survival or composite clinical outcomes. The rMRS was better than the Amsterdam\u2013Oxford model for predicting 1-year transplant-free survival, c-statistics 0.75 and 0.70, respectively. The UK-PSC score outperformed the rMRS for 10-year transplant-free survival, c-statistics 0.85 and 0.69, respectively. An enhanced liver fibrosis score was independently associated with transplant-free survival after adjusting for rMRS. PREsTo predicts 5-year hepatic decompensation with a c-statistic modestly higher than rMRS; 0.90 and 0.85, respectively. Conclusion: Newer prognostic models, including the UK-PSC score and PREsTo, are more accurate at predicting clinical endpoints in PSC compared to the rMRS. Time frames and clinical endpoints are not standard among studies.<\/jats:p>","DOI":"10.3390\/jcm10194476","type":"journal-article","created":{"date-parts":[[2021,9,28]],"date-time":"2021-09-28T21:38:09Z","timestamp":1632865089000},"page":"4476","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Systematic Review of Prognostic Models Compared to the Mayo Risk Score for Primary Sclerosing Cholangitis"],"prefix":"10.3390","volume":"10","author":[{"given":"Paul A.","family":"Schmeltzer","sequence":"first","affiliation":[{"name":"Division of Hepatology Atrium Health, Wake Forest University School of Medicine, Charlotte, NC 28204, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark W.","family":"Russo","sequence":"additional","affiliation":[{"name":"Division of Hepatology Atrium Health, Wake Forest University School of Medicine, Charlotte, NC 28204, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,9,28]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1053\/j.gastro.2009.11.046","article-title":"Genome-Wide Association Analysis in Primary Sclerosing Cholangitis","volume":"138","author":"Karlsen","year":"2010","journal-title":"Gastroenterolgy"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"2045","DOI":"10.1002\/hep.26565","article-title":"Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis","volume":"58","author":"Boonstra","year":"2013","journal-title":"Hepatology"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1357","DOI":"10.1002\/hep.28256","article-title":"Surrogate End-points for Clinical Trials in Primary Sclerosing Cholangitis: Review and Results From an International PSC Study Group Consensus Process","volume":"63","author":"Ponsioen","year":"2016","journal-title":"Hepatology"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1111\/apt.16296","article-title":"Systematic review: Non-invasive prognostic tests for primary sclerosing cholangitis","volume":"53","author":"Mazhar","year":"2021","journal-title":"Aliment. Pharmacol. Ther."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"2008","DOI":"10.1001\/jama.283.15.2008","article-title":"Meta-analysis of Observational Studies in EpidemiologyA Proposal for Reporting","volume":"283","author":"Stroup","year":"2000","journal-title":"JAMA"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"W1","DOI":"10.7326\/M18-1377","article-title":"PROBAST: A Tool to Assess Risk of Bias and Applicability of Prediction Model Studies: Explanation and Elaboration","volume":"170","author":"Moons","year":"2019","journal-title":"Ann. Intern. Med."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1016\/S0025-6196(11)64614-4","article-title":"A Revised Natural History Model for Primary Sclerosing Cholangitis","volume":"75","author":"Kim","year":"2000","journal-title":"Mayo Clin. Proc."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1864","DOI":"10.1136\/gutjnl-2016-313681","article-title":"A novel prognostic model for transplant-free survival in primary sclerosing cholangitis","volume":"67","author":"Wang","year":"2018","journal-title":"Gut"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"992","DOI":"10.1016\/j.jhep.2019.06.012","article-title":"Validation, clinical utility and limitations of the Amsterdam-Oxford model for primary sclerosing cholangitis","volume":"71","author":"Goet","year":"2019","journal-title":"J. Hepatol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1002\/hep.27825","article-title":"Enhanced Liver Fibrosis Predicts Trans-plant-Free Survival in Primary Sclerosing Cholangitis","volume":"62","author":"Vesterhus","year":"2015","journal-title":"Hepatology"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"e13088","DOI":"10.1111\/eci.13088","article-title":"Cholangiocarcinoma is associated with a raised enhanced liver fibrosis score independent of primary sclerosing cholangitis","volume":"49","author":"Saffioti","year":"2019","journal-title":"Eur. J. Clin. Investig."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"2120","DOI":"10.1002\/hep.30479","article-title":"Factors Associated With Outcomes of Patients With Primary Sclerosing Cholangitis and Development and Validation of a Risk Scoring System","volume":"69","author":"Goode","year":"2019","journal-title":"Hepatology"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1002\/hep.30085","article-title":"Primary Sclerosing Cholangitis Risk Estimate Tool (PREsTo) Predicts Outcomes of the Disease: A Derivation and Validation Study Using Machine Learning","volume":"71","author":"Eaton","year":"2020","journal-title":"Hepatology"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1002\/hep.1840100406","article-title":"Primary sclerosing cholangitis: Natural history, prognostic factors and survival analysis","volume":"10","author":"Wiesner","year":"1989","journal-title":"Hepatology"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1016\/j.jhep.2012.10.013","article-title":"Improvement of serum alkaline phosphatase to <1.5 upper limit of normal predicts better outcome and reduced risk of cholangiocarcinoma in primary sclerosing cholangitis","volume":"58","author":"Djordjevic","year":"2013","journal-title":"J. Hepatol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1292","DOI":"10.1111\/apt.12979","article-title":"Reduction in alkaline phosphatase is associated with longer survival in primary sclerosing cholangitis, independent of dominant stenosis","volume":"40","author":"Rupp","year":"2014","journal-title":"Aliment. Pharmacol. Ther."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1002\/hep.23082","article-title":"High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis","volume":"50","author":"Lindor","year":"2009","journal-title":"Hepatology"},{"key":"ref_18","first-page":"1576","article-title":"Changes in Liver Stiffness, Measured by Magnetic Resonance Elastography, Associated with Hepatic Decompensation in Patients With Primary Sclerosing Cholangitis. Clin. Gastroenterol","volume":"18","author":"Eaton","year":"2020","journal-title":"Hepatology"},{"key":"ref_19","first-page":"2785","article-title":"Simple Magnetic Resonance Scores Associate with Outcomes of Patients With Primary Sclerosing Cholangitis. Clin. Gastroenterol","volume":"17","author":"Lemoinne","year":"2019","journal-title":"Hepatology"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"30847","DOI":"10.1038\/srep30847","article-title":"Serum Autotaxin is a Marker of the Severity of Liver Disease and Overall Survival in Patients with Cholestatic Liver Diseases","volume":"6","author":"Wunsch","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"145","DOI":"10.15403\/jgld.2014.1121.272.cho","article-title":"Serum mi-RNA is an Independent Biomarker of Survival in Patients with Primary Sclerosing Cholangitis","volume":"27","author":"Friedrich","year":"2018","journal-title":"J. Gastro Liver Dis."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/10\/19\/4476\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T07:06:31Z","timestamp":1760166391000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/10\/19\/4476"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,9,28]]},"references-count":21,"journal-issue":{"issue":"19","published-online":{"date-parts":[[2021,10]]}},"alternative-id":["jcm10194476"],"URL":"https:\/\/doi.org\/10.3390\/jcm10194476","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,9,28]]}}}